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1.
Transplant Proc ; 56(3): 588-595, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38521737

RESUMEN

BACKGROUND: Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of chronic liver disease, which develops insidiously as a result of chronic liver disease. The prognosis for untreated patients with HPS is extremely poor, and liver transplantation (LT) serves as the only effective means for treating this condition. Here, we performed a retrospective analysis to evaluate the efficacy of LT on the survival and long-term prognosis of patients with HPS. METHODS: Clinical data, including survival and postoperative efficacy, from patients with HPS from records as obtained over the period from January 1 to December 31, 2022. All records were from a waiting list for LT at the Beijing Friendship Hospital Affiliated with Capital Medical University. RESULTS: Among the 274 patients on the LT waiting list, 37 were diagnosed with HPS (13.50%) and were enrolled. Survival rates of patients with HPS receiving an LT were greater, whereas a statistically significant difference was obtained between patients with LT vs non-LT with moderate to severe HPS (P = .003). The overall time until death without LT was 4-72 days after their initial HPS diagnosis. Patients with HPS receiving an LT showed a significant improvement in the state of oxygenation after surgery (P = .001). CONCLUSION: Comprehensive preoperative screening of patients on the waiting list for LT is critical to identify those patients with HPS who would maximally benefit from LT. Survival rates of patients with moderate to severe HPS are significantly increased after LT, a procedure that should be performed as soon as possible in these patients with HPS.


Asunto(s)
Síndrome Hepatopulmonar , Trasplante de Hígado , Humanos , Síndrome Hepatopulmonar/cirugía , Síndrome Hepatopulmonar/mortalidad , Estudios Retrospectivos , Femenino , Masculino , Persona de Mediana Edad , Resultado del Tratamiento , Adulto , Listas de Espera , Tasa de Supervivencia
2.
Clin Transplant ; 38(1): e15171, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-37897208

RESUMEN

BACKGROUND & AIMS: Concomitant respiratory disease is a common finding in patients with hepatopulmonary syndrome (HPS). Among patients who underwent liver transplantation (LT) for HPS, we compared characteristics and outcome of patients with versus without concomitant respiratory disease. METHODS: This single center retrospective observational study included patients with HPS who underwent LT between 1999 and 2020. RESULTS: During the study period, 32 patients with HPS received a LT; nine (28%) with concomitant respiratory disease of whom one required a combined lung-liver transplantation. Patients with concomitant respiratory disease had higher PaCO2 (38 vs. 33 mm Hg, p = .031). The 30-day postoperative mortality was comparable, but the estimated cumulative probability of resolution of oxygen therapy after LT in HPS patients with versus those without concomitant respiratory disease was lower: 63% versus 91% at 12 months and 63% versus 100% at 18 months (HR 95% CI .140-.995, p = .040). In addition to the presence of concomitant respiratory disease (p = .040), history of smoking (p = .012), and high baseline 99mTcMAA shunt fraction (≥20%) (p = .050) were significantly associated with persistent need of oxygen therapy. The 5-year estimated cumulative probability of mortality in patients with concomitant respiratory disease was worse: 50% versus 23% (HR 95% CI .416-6.867, p = .463). CONCLUSIONS: The presence of a concomitant respiratory disease did not increase the short-term postoperative mortality after LT in patients with HPS. However, it resulted in a longer need for oxygen therapy.


Asunto(s)
Síndrome Hepatopulmonar , Trasplante de Hígado , Humanos , Síndrome Hepatopulmonar/cirugía , Síndrome Hepatopulmonar/complicaciones , Trasplante de Hígado/efectos adversos , Pulmón , Oxígeno , Terapia por Inhalación de Oxígeno , Estudios Retrospectivos
3.
Probl Endokrinol (Mosk) ; 67(5): 58-66, 2021 10 03.
Artículo en Ruso | MEDLINE | ID: mdl-34766492

RESUMEN

We describe a 15-year girl, who developed panhypopituitarism and diencephalic obesity after surgical excision of craniopharyngioma, followed by nonalcoholic fatty liver disease and cirrhosis 5 years after surgery. Cirrhosis in this case manifested by hypoxia due to hepatopulmonary syndrome, and despite cure of craniopharyngioma by surgery and radiosurgery treatment and adequate hormonal substitution therapy patient died 9 years after surgery. Growth hormone substitutional therapy in patients with hypopituitarism, and steatohepatitis may decrease liver triglyceride accumulation and prevent end-stage liver disease.


Asunto(s)
Craneofaringioma , Síndrome Hepatopulmonar , Enfermedad del Hígado Graso no Alcohólico , Neoplasias Hipofisarias , Craneofaringioma/complicaciones , Craneofaringioma/cirugía , Femenino , Síndrome Hepatopulmonar/complicaciones , Síndrome Hepatopulmonar/cirugía , Humanos , Cirrosis Hepática/complicaciones , Cirrosis Hepática/cirugía , Enfermedad del Hígado Graso no Alcohólico/complicaciones , Enfermedad del Hígado Graso no Alcohólico/cirugía , Obesidad/complicaciones , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/cirugía
5.
Pediatr Transplant ; 25(2): e13802, 2021 03.
Artículo en Inglés | MEDLINE | ID: mdl-32777145

RESUMEN

DC is caused by defects at the level of telomere maintenance, and cells from patients with this disease have abnormally short telomeres and show premature senescence. One consequence of DC is bone marrow failure. Thus, patients with DC often require HSCT. However, HSCT does not ameliorate other DC-related manifestations. In fact, HSCT can accelerate organ dysfunction due to treatment-related complications, and solid organ transplantation is required in some patients with DC. In this report, we describe the clinical course of a 5-year-old boy who was transferred to our hospital because of progressive dyspnea, 2 years after HSCT. At admission, he had tachypnea and hypoxemia. A liver biopsy was performed for suspected HPS caused by PH, and LT was considered. Eventually, his hypoxemia worsened, and he was transferred to a PICU and started on VA ECMO. He subsequently underwent a CLLT. ECMO was stopped on post-operative day 12, extubation was achieved on post-operative day 29, and the patient recovered well from the surgery. Our results show that CLLT could be a life-saving treatment option for DC patients with very severe HPS in whom a poor outcome is expected after LT.


Asunto(s)
Disqueratosis Congénita/complicaciones , Síndrome Hepatopulmonar/cirugía , Hipertensión Portal/cirugía , Trasplante de Hígado/métodos , Trasplante de Pulmón/métodos , Preescolar , Síndrome Hepatopulmonar/etiología , Humanos , Hipertensión Portal/etiología , Masculino , Gravedad del Paciente
6.
Clin Liver Dis ; 24(3): 373-388, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32620278

RESUMEN

Transjugular intrahepatic portosystemic shunts is an established treatment for portal hypertensive complications. Advancements in technology and technique have led to novel indications, including treatment of chronic portal vein thrombosis and use before abdominal surgery to alleviate portal hypertensive complications. Use of TIPS can facilitate the embolization of large portal-systemic shunts to alleviate refractory hepatic encephalopathy owing to excessive portal shunting. Despite these advances, transjugular intrahepatic portosystemic shunts is an invasive procedure with risk for complications and should be performed at a center with expertise to ensure a successful patient outcome.


Asunto(s)
Hepatopatías/cirugía , Vena Porta , Derivación Portosistémica Intrahepática Transyugular/métodos , Trombosis/cirugía , Ascitis/cirugía , Enfermedad Crónica , Contraindicaciones de los Procedimientos , Síndrome Hepatopulmonar/cirugía , Síndrome Hepatorrenal/cirugía , Humanos , Hipertensión Portal/cirugía , Selección de Paciente , Derivación Portosistémica Intrahepática Transyugular/efectos adversos , Stents , Fístula Vascular/cirugía
7.
Pediatr Transplant ; 24(3): e13695, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32166868

RESUMEN

Short telomere syndromes are a heterogenous spectrum of disorders leading to premature cellular aging. These may involve bone marrow failure, adult-onset idiopathic pulmonary fibrosis, and liver disease, and classical entities such as dyskeratosis congenita. We report a patient who presented with common variable immunodeficiency at 3 years of age and autoimmune cytopenias at 8 years of age. He was found to have short telomeres, and genetic testing confirmed a hemizygous mutation NM_001363.4: c.-142C > G in DKC1 gene. He subsequently developed cirrhosis with severe portal hypertension and hepatopulmonary syndrome, prompting liver transplantation at 11 years of age. He remains well 10 years after transplant with no progression of bone marrow failure or progressive lung disease. In conclusion, short telomere syndromes should be considered as a potential cause of pediatric liver disease of unknown etiology, and in severe cases, isolated liver transplantation may be both appropriate and successful.


Asunto(s)
Proteínas de Ciclo Celular/genética , Fallo Renal Crónico/cirugía , Trasplante de Hígado , Mutación , Proteínas Nucleares/genética , Acortamiento del Telómero/genética , Trastornos de Fallo de la Médula Ósea , Niño , Marcadores Genéticos , Síndrome Hepatopulmonar/etiología , Síndrome Hepatopulmonar/cirugía , Humanos , Fallo Renal Crónico/etiología , Cirrosis Hepática/etiología , Cirrosis Hepática/cirugía , Masculino , Síndrome
8.
Rev. gastroenterol. Perú ; 38(3): 242-247, jul.-set. 2018. ilus, tab
Artículo en Español | LILACS | ID: biblio-1014090

RESUMEN

Introducción: El síndrome hepatopulmonar (SHP) es una complicación grave de la enfermedad hepática, la cual se caracteriza por la presencia de vasodilatación intrapulmonar e hipoxemia progresiva, siendo el trasplante de hígado el único tratamiento efectivo. Objetivo: Mostrar nuestros resultados de los pacientes con síndrome hepatopulmonar sometidos a trasplante hepático. Materiales y métodos: Estudio retrospectivo, descriptivo y trasversal. Desde marzo del 2000 a diciembre del 2016 se realizaron 226 trasplantes de hígado. Del total, se excluyeron a 25 pacientes: 12 retrasplantes, 9 trasplantes dobles higadoriñon, 2 trasplantes con falla hepática aguda, 2 trasplantes en pacientes no cirróticos. De los 201 pacientes con diagnóstico pretrasplante de cirrosis hepática, 19 tuvieron criterios de SHP; quienes fueron distribuidos según edad, sexo, nivel de hipoxemia (pO2), score CHILD, score MELD. La reversibilidad de la hipoxemia post trasplante se midió con una cutt off de p0(2) >75 mmHg. Resultados: La prevalencia del SHP en nuestra serie fue 9,45%. La edad promedio fue 41 años (14-65); la relación M/F de 1,65. El 78,94% (15/19) fueron adultos. 89,5% (17/19) fueron score de CHILD B y C, y el 68,4% tuvieron SHP severo y muy severo. En el 94,11% de los pacientes se demostró reversibilidad del SHP. La tasa de mortalidad temprana en los pacientes con SHP fue 10,4%. Conclusiones: La prevalencia del SHP fue del 9,45%. Los pacientes trasplantados con y sin SHP tuvieron similar sobrevida.


Introduction: Hepatopulmonary syndrome (HPS) is a serious complication of liver disease, which is characterized by the presence of intrapulmonary vasodilation and progressive hypoxemia. Liver transplantation is the only effective treatment. Objective: To show our results of patients with hepatopulmonary syndrome undergoing liver transplantation. Materials and methods: Retrospective, descriptive and cross-sectional study. From March 2000 to December 2016; 226 liver transplants were performed. Of the total, 25 patients were excluded: 12 retransplantation, 9 liver-kidney combined transplants, 2 transplants for acute liver failure, 2 transplants in non-cirrhotic patients. Of the 201 patients with pretransplant diagnosis of liver cirrhosis, 19 filled criteria for SHP; who were distributed according to age, sex, hypoxemia level (pO2), Child-Pugh score and MELD score. The reversibility hypoxemia after liver trasplantation was measured with a cut-off of p0(2) >75 mmHg. Results: The prevalence of SHP in our series was 9.45%. The average age was 41 years (14-65); the M / F ratio of 1.65. The 78.94% (15/19) were adults. 89.5% (17/19) were Score of Child-Pugh B and C, and 68.4% had severe and very severe SHP. In 94.11% of patients, reversibility SHP founded. The early mortality rate (30 days) in patients with SHP was 10.4%. Conclusions: The prevalence of HPS in our series was 9.45%. Transplanted patients with and without SHP had similar survival.


Asunto(s)
Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Trasplante de Hígado , Síndrome Hepatopulmonar/cirugía , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/epidemiología , Índice de Severidad de la Enfermedad , Estudios Transversales , Estudios Retrospectivos , Trasplante de Riñón/estadística & datos numéricos , Hepatitis Autoinmune/cirugía , Síndrome Hepatopulmonar/epidemiología , Enfermedad del Hígado Graso no Alcohólico/cirugía , Utilización de Procedimientos y Técnicas , Departamentos de Hospitales/estadística & datos numéricos , Hospitales Públicos/estadística & datos numéricos , Cirrosis Hepática/cirugía , Hipoxia/etiología , Hipoxia/epidemiología
9.
Orphanet J Rare Dis ; 13(1): 69, 2018 05 02.
Artículo en Inglés | MEDLINE | ID: mdl-29716638

RESUMEN

Vitamin A intoxication is a rare cause of liver disease, but the risk increases in patients with underlying liver dysfunction. We present a patient with Shwachman-Diamond Syndrome who developed liver fibrosis, portal hypertension and very severe hepatopulmonary syndrome as a consequence of chronic vitamin A intoxication. She underwent successful liver transplantation with complete resolution of the pulmonary shunting.


Asunto(s)
Enfermedades de la Médula Ósea/cirugía , Insuficiencia Pancreática Exocrina/cirugía , Síndrome Hepatopulmonar/etiología , Síndrome Hepatopulmonar/cirugía , Lipomatosis/cirugía , Hepatopatías/complicaciones , Trasplante de Hígado/métodos , Vitamina A/toxicidad , Niño , Preescolar , Femenino , Humanos , Hepatopatías/etiología , Síndrome de Shwachman-Diamond
10.
Einstein (Säo Paulo) ; 15(3): 322-326, July-Sept. 2017. tab
Artículo en Inglés | LILACS | ID: biblio-891389

RESUMEN

ABSTRACT Objective To compare mechanical ventilation time, need for non-invasive ventilation, length of intensive care unit stay, and hospital stay after liver transplant in cirrhotic patients with and with no diagnosis of hepatopulmonary syndrome. Methods This was a prospective cohort study with a convenience sample of 178 patients (92 with hepatopulmonary syndrome) who were diagnosed as alcoholic or hepatitis C virus cirrhosis. The statistical analysis included Kolmogorov-Smirnov test and Students t test. Data were analyzed using SPSS version 16.0, and p values <0.05 were considered significant. Results Out of 178 patients, 90 underwent transplant (48 with no hepatopulmonary syndrome). The Group diagnosed with Hepatopulmonary Syndrome had longer mechanical ventilation time (19.5±4.3 hours versus 12.5±3.3 hours; p=0.02), an increased need for non-invasive ventilation (12 versus 2; p=0.01), longer intensive care unit stay (6.7±2.1 days versus 4.6±1.5 days; p=0.02) and longer hospital stay (24.1±4.3 days versus 20.2±3.9 days; p=0.01). Conclusion Cirrhotic patients Group diagnosed with Hepatopulmonary Syndrome had higher mechanical ventilation time, more need of non-invasive ventilation, as well as longer intensive care unit and hospital stay.


RESUMO Objetivo Comparar tempo de ventilação mecânica, necessidade de uso de ventilação não invasiva, tempo de permanência na unidade de terapia intensiva e tempo de hospitalização após transplante hepático em cirróticos com e sem diagnóstico de síndrome hepatopulmonar. Métodos Estudo de coorte prospectiva com amostra de conveniência composta por 178 pacientes (92 com síndrome hepatopulmonar) com diagnóstico de cirrose por álcool ou pelo vírus da hepatite C. A análise estatística foi realizada por meio do teste Kolmogorov-Smirnov e do teste t de Student. Os dados foram analisados pelo programa SPSS versão 16.0, e valores de p<0,05 foram considerados significantes. Resultados Dos 178 pacientes, 90 foram transplantados (48 sem síndrome hepatopulmonar). O Grupo com Síndrome Hepatopulmonar apresentou maior tempo de ventilação mecânica (19,5±4,3 horas versus 12,5±3,3 horas; p=0,02), maior necessidade de uso de ventilação não invasiva (12 versus 2; p=0,01), maior permanência na unidade de terapia intensiva (6,7±2,1 dias versus 4,6±1,5 dias; p=0,02) e maior tempo de hospitalização (24,1±4,3 dias versus 20,2±3,9 dias; p=0,01). Conclusão O Grupo com Síndrome Hepatopulmonar apresentou maiores tempo de ventilação mecânica, necessidade de uso de ventilação não invasiva, permanência na unidade de terapia intensiva e tempo de hospitalização.


Asunto(s)
Humanos , Masculino , Femenino , Respiración Artificial/estadística & datos numéricos , Trasplante de Hígado , Síndrome Hepatopulmonar/cirugía , Tiempo de Internación/estadística & datos numéricos , Cirrosis Hepática/cirugía , Factores de Tiempo , Estudios Prospectivos , Persona de Mediana Edad
11.
J Clin Pharm Ther ; 42(5): 646-648, 2017 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-28543822

RESUMEN

WHAT IS KNOWN AND OBJECTIVE: Limited data exist surrounding the metabolism and safety of garlic supplements. CASE DESCRIPTION: A patient with a history of hepatopulmonary syndrome (HPS) and orthotopic liver transplantation was admitted to our surgery transplant service with severe hypoxaemia. The patient was started on high-dose Garlicin Cardio® (Allium sativum) for HPS and soon after had elevated liver function tests. Garlicin Cardio® was discontinued and liver enzymes normalized. A liver biopsy revealed mild periportal cholestatic reaction suggesting potential drug-induced aetiology. WHAT IS NEW AND CONCLUSION: This is the first description of liver injury secondary to garlic supplementation. Therefore, this garlic supplement should be listed as a potential cause of acute drug-induced liver injury.


Asunto(s)
Enfermedad Hepática Inducida por Sustancias y Drogas/etiología , Suplementos Dietéticos/efectos adversos , Ajo/efectos adversos , Trasplante de Hígado , Adulto , Ajo/química , Síndrome Hepatopulmonar/cirugía , Humanos , Pruebas de Función Hepática , Masculino
12.
Pediatr Transplant ; 21(2)2017 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-28078819

RESUMEN

A 15-year-old boy first presented with severe lung lesions and hypoxia and he was considered as a lung transplant candidate. Upon evaluation, hepatopulmonary syndrome, multiple nodular liver lesions, and Abernethy type 1b malformation were diagnosed. The patient underwent successful right lobe live donor liver transplantation, and all of the symptoms disappeared soon after the transplant. He is currently alive and well with excellent liver and lung functions 4 years after surgery.


Asunto(s)
Síndrome Hepatopulmonar/cirugía , Hepatopatías/cirugía , Fallo Hepático/cirugía , Trasplante de Hígado , Donadores Vivos , Vena Porta/anomalías , Adolescente , Síndrome Hepatopulmonar/complicaciones , Humanos , Hepatopatías/complicaciones , Masculino , Venas Mesentéricas/cirugía , Vena Porta/cirugía , Tomografía Computarizada por Rayos X
13.
Pediatr Transplant ; 20(3): 460-2, 2016 May.
Artículo en Inglés | MEDLINE | ID: mdl-26992455

RESUMEN

HPS has been described in 9-20% of children with end-stage liver disease. We present a case of a previously, asymptomatic nine-yr-old incidentally found to have low oxygen saturation. Physical exam was remarkable for digital clubbing, splenomegaly and orthodeoxia. Laboratory evaluation revealed a low platelet count, hyperammonemia, and prolonged coagulation studies. Sonography showed evidence of splenomegaly and portal venous hypertension. High resolution CT thorax and CTA were normal. HPS was confirmed by agitated saline contrast enhanced echocardiography and Tc-99m MAA scan with evidence of intrapulmonary vascular dilatations. Liver biopsy was performed and consistent with autoimmune hepatitis. A high clinical index of suspicion should be maintained for HPS in pediatric patients who have unexplained hypoxemia as typical signs and symptoms of severe liver disease are often absent. In this report, we discuss a case of HPS complicated AIH in a pediatric patient and review the relevant literature.


Asunto(s)
Enfermedad Hepática en Estado Terminal/complicaciones , Enfermedad Hepática en Estado Terminal/cirugía , Hepatitis Autoinmune/cirugía , Síndrome Hepatopulmonar/cirugía , Biopsia , Coagulación Sanguínea , Niño , Medios de Contraste/química , Ecocardiografía , Hepatitis Autoinmune/complicaciones , Síndrome Hepatopulmonar/complicaciones , Humanos , Hipertensión Portal/complicaciones , Hipoxia/complicaciones , Hígado/patología , Masculino , Oxígeno/química , Vena Porta/fisiopatología , Esplenomegalia/complicaciones , Compuestos de Sulfhidrilo/química , Agregado de Albúmina Marcado con Tecnecio Tc 99m/química
14.
Gastroenterol Hepatol ; 38(6): 398-408, 2015.
Artículo en Español | MEDLINE | ID: mdl-25840463

RESUMEN

Hepatopulmonary syndrome is characterized by the presence of liver disease, pulmonary vascular dilatations, and arterial hypoxemia. It is usually associated with cirrhosis of any origin, but has been described in other liver diseases, both acute and chronic, and not always associated with portal hypertension. The gold standard method to detect pulmonary vascular dilations is contrast enhancement echocardiography with saline and is essential for the diagnosis of hepatopulmonary syndrome. These dilatations reflect changes in the pulmonary microvasculature (vasodilatation, intravascular monocyte accumulation, and angiogenesis) and induce a ventilation/perfusion mismatch, or even true intrapulmonary shunts, which eventually trigger hypoxemia. This syndrome worsens patients' prognosis and impairs their quality of life and may lead to the need for liver transplantation, which is the only effective and definitive treatment. In this article, we review the etiological, pathophysiological, clinical and therapeutic features of this syndrome.


Asunto(s)
Síndrome Hepatopulmonar , Análisis de los Gases de la Sangre , Terapia Combinada , Diagnóstico Diferencial , Ecocardiografía/métodos , Síndrome Hepatopulmonar/diagnóstico por imagen , Síndrome Hepatopulmonar/epidemiología , Síndrome Hepatopulmonar/etiología , Síndrome Hepatopulmonar/fisiopatología , Síndrome Hepatopulmonar/cirugía , Humanos , Hipertensión Portal/complicaciones , Hipertensión Portal/fisiopatología , Hipoxia/etiología , Hepatopatías/complicaciones , Hepatopatías/fisiopatología , Trasplante de Hígado , Terapia por Inhalación de Oxígeno , Pronóstico , Circulación Pulmonar , Calidad de Vida , Cintigrafía , Radiofármacos , Agregado de Albúmina Marcado con Tecnecio Tc 99m , Vasodilatación
15.
Duodecim ; 130(16): 1628-32, 2014.
Artículo en Finés | MEDLINE | ID: mdl-25269369

RESUMEN

Hepatopulmonary syndrome (HPS) is characterized by the triad of liver disease, intrapulmonary vascular dilatation and arterial deoxygenation. Mediating factors are tumor necrosis factor a, endothelin 1 and nitric oxide. Typical symptom is an increase in dyspnea while in standing position, orthodeoxia. In orthodeoxia hypoxemia accentuates while the partial pressure of oxygen in arterial blood decreases by 5% or more. The blood flow distribution to the lungs is changed as the pulmonary vascular tone is altered. Diagnosis is based on the evidence of liver disease, hypoxemia and pulmonary vascular shunt detected by the so-called bubble test. Liver transplantation is currently the only efficient therapeutic option.


Asunto(s)
Síndrome Hepatopulmonar/diagnóstico , Diagnóstico Diferencial , Síndrome Hepatopulmonar/fisiopatología , Síndrome Hepatopulmonar/cirugía , Humanos , Hipoxia/diagnóstico , Hipoxia/fisiopatología , Trasplante de Hígado , Postura/fisiología
16.
Lung ; 192(3): 421-7, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24682307

RESUMEN

Hepatopulmonary syndrome (HPS) is characterized by impaired oxygenation due to pulmonary vascular dilatation in patients with end-stage liver disease. At our center, we identified 29 patients who were listed for liver transplantation (LT) with a model for end-stage liver disease exception for HPS between 2001 and 2012. Five of these patients were found to have concurrent interstitial lung disease (ILD). The chest high-resolution computed-tomography demonstrated ground-glass opacities and subpleural reticulation, most consistent with nonspecific interstitial pneumonia (NSIP). All four of our patients who underwent LT experienced prolonged hypoxemia postoperatively, with one surgery-related death. However, the three surviving patients had eventual resolution of their hypoxemia with no evidence of ILD progression. In conclusion, we report a high prevalence of ILD, most consistent with NSIP, among patients with HPS. Although there may be increased perioperative risks, the finding of ILD in patients with HPS should not be considered an absolute contraindication to LT.


Asunto(s)
Síndrome Hepatopulmonar/complicaciones , Enfermedades Pulmonares Intersticiales/complicaciones , Adulto , Anciano , Biopsia , California , Resultado Fatal , Femenino , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/cirugía , Humanos , Hipoxia/diagnóstico , Hipoxia/etiología , Trasplante de Hígado/efectos adversos , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Selección de Paciente , Medición de Riesgo , Factores de Riesgo , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Listas de Espera
17.
World J Gastroenterol ; 20(10): 2586-94, 2014 Mar 14.
Artículo en Inglés | MEDLINE | ID: mdl-24627594

RESUMEN

Hepatopulmonary syndrome (HPS) is characterized as a triad: liver disease, intrapulmonary vascular dilatation and arterial hypoxemia. HPS is reported to be present in 4% to 32% of adult patients with end-stage liver disease and in 9%-20% of children. The pathogenesis of HPS has not been clearly identified. Portal hypertension causes impairment in the perfusion of the bowel and increases the enteral translocation of Gram (-) bacteria and endotoxins. This stimulates the release of vasoactive mediators, such as tumor necrosis factor-alpha, heme oxygenase-derived carbon monoxide and nitric oxide. Genetic alterations have not been associated with this syndrome yet; however, cytokines and chemokines have been suggested to play a role. Recently, it was reported that cumulated monocytes lead to the activation of vascular endothelial growth factor-dependent signaling pathways and pulmonary angiogenesis, which plays an important role in HPS pathogenesis. At present, the most effective and only radical treatment is a liver transplant (LT). Cirrhotic patients who are on the waiting list for an LT have a shorter survival period if they develop HPS. Therefore, it is suggested that all cirrhotic cases should be followed closely for HPS and they should have priority in the waiting list.


Asunto(s)
Síndrome Hepatopulmonar/etiología , Cirrosis Hepática/complicaciones , Animales , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/fisiopatología , Síndrome Hepatopulmonar/cirugía , Humanos , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/cirugía , Trasplante de Hígado , Selección de Paciente , Valor Predictivo de las Pruebas , Pronóstico , Medición de Riesgo , Factores de Riesgo , Listas de Espera
18.
Arq Bras Cir Dig ; 27(1): 56-8, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24676301

RESUMEN

BACKGROUND: Hepatopulmonary syndrome is formed by a triad of liver disease, intrapulmonary vascular dilatation and changes in blood gases. This condition is present in 4-32% of patients with cirrhosis. AIM: To analyze the blood gas changes data of patients in liver-transplant waiting list. METHOD: Clinical data of 279 patients in liver transplantation waiting list in May 2013 were studied. Overall patient was analyzed by the demographic aspects, laboratorial and image findings on exams that determine lung disease (hypoxemia) in these cirrhotic patients. The mean values and standard deviations were used to examine normally distributed variables. RESULTS: There was a high prevalence of male patients (68%); the mean age was 51(± 5,89) years, and the predominant reason for listing was hepatitis C cirrhosis. The MELD score mean was 16 ± 5,89, without prioritization or special situation. The most common blood type was O in 129 cases (46%) and the mean of body max index was 25,94 ± 4,58. Regarding arterial blood gas tests was observed 214 patients with PaO2 <90 mmHg, 80 with PaO2 <80 mmHg and 39 with PaO2 <50 mmHg. In relation to O2 saturation, 50 patients had <90%, 33 <80% and 10 <50%. CONCLUSION: Was observed a high rate of hypoxemia in patients on waiting list liver transplant. Due to the high severity and morbidity, is suggested better monitoring and therapeutic support to hypoxemic patients on liver transplant waiting list.


Asunto(s)
Síndrome Hepatopulmonar/complicaciones , Hipoxia/epidemiología , Hipoxia/etiología , Trasplante de Hígado , Análisis de los Gases de la Sangre , Femenino , Síndrome Hepatopulmonar/sangre , Síndrome Hepatopulmonar/cirugía , Humanos , Hipoxia/sangre , Cirrosis Hepática , Masculino , Persona de Mediana Edad , Listas de Espera
19.
ABCD (São Paulo, Impr.) ; 27(1): 56-58, Jan-Mar/2014. graf
Artículo en Inglés | LILACS | ID: lil-703978

RESUMEN

Background: Hepatopulmonary syndrome is formed by a triad of liver disease, intrapulmonary vascular dilatation and changes in blood gases. This condition is present in 4-32% of patients with cirrhosis. Aim : To analyze the blood gas changes data of patients in liver-transplant waiting list. Method: Clinical data of 279 patients in liver transplantation waiting list in May 2013 were studied. Overall patient was analyzed by the demographic aspects, laboratorial and image findings on exams that determine lung disease (hypoxemia) in these cirrhotic patients. The mean values and standard deviations were used to examine normally distributed variables. Results: There was a high prevalence of male patients (68%); the mean age was 51(±5,89) years, and the predominant reason for listing was hepatitis C cirrhosis. The MELD score mean was 16±5,89, without prioritization or special situation. The most common blood type was O in 129 cases (46%) and the mean of body max index was 25,94±4,58. Regarding arterial blood gas tests was observed 214 patients with PaO2 <90 mmHg, 80 with PaO2 <80 mmHg and 39 with PaO2 <50 mmHg. In relation to O2 saturation, 50 patients had <90%, 33 <80% and 10 <50%. Conclusion: Was observed a high rate of hypoxemia in patients on waiting list liver transplant. Due to the high severity and morbidity, is suggested better monitoring and therapeutic support to hypoxemic patients on liver transplant waiting list. .


Racional: A síndrome hepatopulmonar é formada por tríade clínica com doença do fígado, dilatação vascular intrapulmonar e alterações nos gases sanguíneos. Esta condição está presente em 4-32% dos pacientes com cirrose. Objetivo : Analisar as alterações gasométricas nos pacientes em lista de espera de transplante de fígado. Método: Foram estudados dados clínicos de 279 pacientes na lista de espera para transplante hepático em maio de 2013. Foram analisados aspectos demográficos, gasometria arterial e achados de imagem que determinam a doença pulmonar (hipoxemia) nestes pacientes cirróticos. Os valores médios e desvios-padrão foram utilizados para examinar as variáveis ​​normalmente distribuídas. Resultados: Houve alta prevalência de homens (68%); a idade média foi de 51 (±5,89) anos; e a razão predominante para listar para o transplante foi cirrose pelo vírus C. O MELD médio foi de 16±5,89, sem priorização ou situação especial. O tipo de sangue mais comum foi O, 129 casos (46%) e a média do índice de massa corporal foi 25,94±4,58. Com relação aos exames de gasometria arterial, observou-se 214 pacientes com PaO2 <90 mmHg, 80 com PaO2 <80 mmHg e 39 com PaO2 <50 mmHg, e em relação à saturação de O2, 50 pacientes <90%, 33 pacientes <80% e 10 pacientes <50%. Conclusão: Observou-se alta taxa de hipoxemia nos pacientes em lista de transplante de fígado; devido à elevada gravidade e morbidade, sugere-se melhor seguimento e suporte terapêutico aos doentes hipoxêmicos na lista de espera para o transplante de fígado. .


Asunto(s)
Femenino , Humanos , Masculino , Persona de Mediana Edad , Hipoxia/epidemiología , Hipoxia/etiología , Síndrome Hepatopulmonar/complicaciones , Trasplante de Hígado , Hipoxia/sangre , Análisis de los Gases de la Sangre , Síndrome Hepatopulmonar/sangre , Síndrome Hepatopulmonar/cirugía , Cirrosis Hepática , Listas de Espera
20.
Chirurgia (Bucur) ; 108(5): 684-8, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24157113

RESUMEN

BACKGROUND AND AIM: The prevalence of hepatopulmonary syndrome (HPS) in the setting of cirrhosis ranges between 4%-47%. We aimed to detect a correlation between heart and lungs findings on contrast-enhanced ultrasound (CEUS)and pulse-oximetry, in order to early detect HPS and thus to improve patients referral to orthotopic liver transplantation(OLT). METHODS: We considered at risk for HPS all patients with delayed left ventricle (LV) enhancement of the contrast agent,at least 3 systolic beats after it appears in the right ventricle(RV). We correlated CEUS results with pulse-oximetry findings,considering to have a positive HPS diagnosis in all patients with both CEUS findings and SaO2 95%. RESULTS: From 186 patients diagnosed with liver cirrhosis, 56 patients (30.10%) had delayed LV enhancement of the contrast agent. Pulse-oximetry showed alterations, such as SaO2 95%and PaO2 70 mmHg in 62 patients (33.33%). Pearson index showed a good correlation between lung and heart CEUS findings and pulse-oximetry (r=0.99) in HPS diagnosis. CONCLUSIONS. Two non-invasive, simple and rapid methods such as CEUS and pulse-oximetry can easily diagnose HPS, a highly fatal complication of liver cirrhosis, and can also guide the future treatment by speeding up OLT recommendations.


Asunto(s)
Síndrome Hepatopulmonar/diagnóstico por imagen , Síndrome Hepatopulmonar/cirugía , Cirrosis Hepática/complicaciones , Trasplante de Hígado , Tamizaje Masivo , Oximetría , Ultrasonografía/métodos , Adulto , Anciano , Anciano de 80 o más Años , Medios de Contraste , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/epidemiología , Síndrome Hepatopulmonar/etiología , Humanos , Trasplante de Hígado/métodos , Masculino , Tamizaje Masivo/métodos , Persona de Mediana Edad , Oximetría/métodos , Selección de Paciente , Prevalencia , Factores de Riesgo , Rumanía/epidemiología , Sensibilidad y Especificidad , Índice de Severidad de la Enfermedad , Resultado del Tratamiento
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